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Heart failure and cardiomyopathies
Original research
Long-term incidence of implantable cardioverter-defibrillator therapy in patients with hypertrophic cardiomyopathy: analysis of appropriate and inappropriate interventions
  1. Emma Basse Christensen1,
  2. Christoffer Rasmus Vissing1,
  3. Elvira Silajdzija1,
  4. Helen Lamiokor Mills1,
  5. Jens Jakob Thune2,3,
  6. Charlotte Larroudé4,
  7. Helle Skovmand Bosselmann5,
  8. Berit Thornvig Philbert1,
  9. Anna Axelsson Raja1,
  10. Alex Hørby Christensen1,2,4,
  11. Henning Bundgaard1,2
  1. 1The Capital Region’s Unit for Inherited Cardiac Diseases, Department of Cardiology, The Heart Centre, Copenhagen University Hospital-Rigshospitalet, Copenhagen, Denmark
  2. 2Department of Clinical Medicine, University of Copenhagen, Copenhagen, Denmark
  3. 3Department of Cardiology, Copenhagen University Hospital - Bispebjerg-Frederiksberg Hospital, Copenhagen, Denmark
  4. 4Department of Cardiology, Copenhagen University Hospital - Herlev-Gentofte Hospital, Copenhagen, Denmark
  5. 5Department of Cardiology, Zealand University Hospital, Roskilde, Denmark
  1. Correspondence to Dr Emma Basse Christensen; emma.basse.christensen.01{at}regionh.dk

Abstract

Background Treatment with implantable cardioverter-defibrillators (ICDs) effectively prevents sudden cardiac death (SCD) in patients with hypertrophic cardiomyopathy (HCM). Identifying patients most likely to benefit from a primary prevention ICD remains challenging. We aimed to investigate the long-term incidence of ICD therapy in patients with HCM according to SCD-risk at baseline.

Methods The study retrospectively included all patients with HCM treated with an ICD for primary or secondary prevention between 1995 and 2022 in Eastern Denmark. Medical records for each patient were evaluated. Patients were stratified into risk groups according to the European Society of Cardiology HCM Risk-SCD score.

Results We included 208 patients (66% male) with HCM and an ICD for primary (78%) or secondary prevention (22%). During a median 10-year follow-up, 66 patients (32%) received appropriate ICD therapy (antitachycardia pacing and/or shock), while 20 (10%) received inappropriate therapy. Patients with an ICD implanted for secondary prevention were almost twice as likely to receive appropriate therapy compared with patients with an ICD implanted for primary prevention (47% vs 28%, p=0.02). The 5-year cumulative incidences of appropriate shock therapy were 17% in patients with a high HCM Risk-SCD score, 16% in patients with an intermediate-risk score and 6% in patients with a low-risk score. A high-risk score was associated with higher cumulative incidence of appropriate shock therapy (p=0.012).

Conclusion One-third of patients with HCM treated with an ICD experienced appropriate ICD therapy. The HCM-Risk SCD score adequately distinguished between low-risk and high-risk patients among those who underwent ICD implantation. Further improvements of risk-tools are needed to identify a larger proportion of the two-thirds of patients who did not benefit from ICD implantation after 10 years of observation.

  • Cardiomyopathy, Hypertrophic
  • Defibrillators, Implantable
  • Risk Assessment

Data availability statement

Data are available upon reasonable request.

https://doi.org/10.1136/heartjnl-2024-325020

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    Data availability statement

    Data are available upon reasonable request.

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    Footnotes

    • Contributors HB, AHC, AAR and CV were responsible for leading the conceptualisation and design of the study. All authors contributed to refining the study design and research questions. EBC, ES, and HLM were responsible for data collection. CV and EBC conducted the statistical analysis. All authors contributed to interpreting the data. EBC drafted the initial versions of the manuscript with input from all coauthors. All authors critically reviewed and provided substantial edits to the manuscript drafts. HB, AHC and AAR provided overall leadership for project coordination and administration.HB, AHC, AAR and EBC ensured that all necessary ethical approvals were in place. EBC is the guarantor. EBC takes full responsibility for the completed work and the conduct of the study, maintained access to the data and made the final decision to publish.

    • Funding The Research Foundation at The Heart Center, Rigshospitalet supported this study.AHC is supported by The Independent Research Fund Denmark (Grant 0134-00363B) and The Novo Nordisk Foundation, Denmark (NNF20OC0065799).

    • Competing interests None declared.

    • Patient and public involvement Patients and/or the public were not involved in the design, or conduct, or reporting or dissemination plans of this research.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Supplemental material This content has been supplied by the author(s). It has not been vetted by BMJ Publishing Group Limited (BMJ) and may not have been peer-reviewed. Any opinions or recommendations discussed are solely those of the author(s) and are not endorsed by BMJ. BMJ disclaims all liability and responsibility arising from any reliance placed on the content. Where the content includes any translated material, BMJ does not warrant the accuracy and reliability of the translations (including but not limited to local regulations, clinical guidelines, terminology, drug names and drug dosages), and is not responsible for any error and/or omissions arising from translation and adaptation or otherwise.